Phlebology 2005; 20:63-81.

(The official journal of the Venous Forum of the Royal Society of Medicine and Societas Phlebologica Scandinavica, Members of the Union Internationale de Phlebologie)

B. McDonagh*, T. King*, S. Sorenson*, A. Cohen*, T. Eaton*, D. E. Huntley*, S. La Baer*, K. Campbell*, and R.C. Guptan^

*Illinois Phlebology Group, Schaumburg, Illinois, USA and ^Department of Clinical Research, Venous Research Foundation, Chicago, Illinois, USA

Objectives: To study the clinical presentation of patients with Klippel-Trenaunay syndrome (KTS) and the management of their venous malformations (VMs)/varicose veins with ultrasound-guided foam sclerotherapy using the compass technique.

Results: The most common presenting complaint (100%) was pain. All but one patient (91%) presented with VMs/varicose veins. Chronic venous insufficiency (CVI) was seen in seven (64%). Soft tissue and bone deformity was present in all (100%). Limb lengthening was seen in eight (73%); limb thickening occurred in six (54%). A very unusual presentation was seen in one patient (9%) who had severe thinning and shortening of the leg. The primary vascular problem was venous malformation and was seen in nine patients (82%). This was followed by capillary malformation (CM)/port wine stain in seven (64%) and isolated lymphatic malformation (LM) in two (18%). The lateral embryonic Klippel-Trenaunay (KT) vein was detected in 10 patients (91%). Deep vein deformity was not detected in any of them. All of the patients had vascular deformities of the lower limb (100%); two (18%) had deformities of the thorax and one (9%) had a gastrointestinal (GI) malformation. None of the patients were aware of a history of KTS in their families; however, five (45%) had a family history of varicose veins. Eight patients (73%) completed the therapeutic plan and had a mean follow-up of 5 ± 3.9 years. Six of these eight patients (75%) felt that they had good-to-excellent results from their treatment. They described their quality of life as being good. Their therapeutic course was uneventful. Follow-up duplex ultrasound evaluation was used to reveal any evolving refluxing tracts that required further sequential treatment.

Conclusions: The results in this series demonstrate the effectiveness of sequential ultrasound-guided foam sclerotherapy using the compass technique in the management of KTS. The chronic nature of the VMs in KTS makes strict clinical monitoring and sequential treatment a necessity.

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